IS case 460: Cortical nephrocalcinosis

Keith Forrest Dockery, MD, MS

University of Rochester

Imaging Sciences URMC 2010
Publication Date: 2010-08-30

History

Patient is a 9-month-old female who initially presented at 3 month of age in the Emergency Department in acuterenal failure, with severe acidemia, hyponatremia, and hyperkalemia, which lead to peritoneal dialysis. Now the patient presented with clinical question of renal dysplasia.

Findings

See images

Diagnosis

Cortical nephrocalcinosis

Discussion

Case discussion: The patient presented initially as a 3 month old with acuterenal failure and severe metabolic derangement, including acidemia, hyponatremia, and hyperkalemia. Hyocalcemia was critical. Severity of the condition lead to peritoneal dialysis.

Topic discussion: Two-thirds of nephrocalcinosis and nephrolithiasis are associated with underlying renal structural defect or infection. Approximately 10% are due to hypercalcemia or hypercalcuria. Approximately 20% are idiopathic.

Two types of ARPKD occur: a juvenile form wherein the liver predominates; and an infantile form wherein renal dysfunction predominates and renal cysts measure 1-2 mm, with ultrasound revealing diffuse echogenicity in cortex and medullary in enlarged kidneys. In the second type, juvenile ARPKD, hepatic fibrosis predominates with hepatosplenomegaly, and variable renal appearance.

In contrast, multicystic dysplastic kidney (MDK) typically presents as a “grape-like” collection of cysts of variable size that do not appear to communicate - cysts measure between 1 mm and 1 cm. MDK is typically unilateral, but can be bilateral in 20%. Failure to find cysts or echogenicity does not eliminated this disorder.

The most common causes of cortical nephrocalcinosis are chronic glomerulonephritis, acute cortical necrosis, and oxalosis. Acute cortical necrosis can be due to ethylene glycol, acute vascular insult, or methoxyflurane anesthesia. Other etiologies of nephrocalcinosis include: infections due to mycobacterium avium-intracellulare, cytomegalovirus, and pneumocystis carinii; Alport’s syndrome, an autosomal dominant hereditary nephritis (deafness and nephropathy).